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9 - Grade/differentiation unknown, not stated, or not applicable
Hypoplastic myelodysplastic syndrome (MDS)
Myelodysplastic syndrome, NOS
Blood: Cytopenias, no blasts Bone marrow: <5% blasts, dysplasia in granulocytes or megakaryocytes
The myelodysplastic syndromes (MDS, formerly known as "preleukemia") are a diverse collection of hematological conditions united by ineffective production of blood cells and varying risks of transformation to acute myelogenous leukemia (AML). Anemia requiring chronic blood transfusion is frequently present.
Myelodysplastic syndrome, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. Further review of the medical record should be done to look for the tests listed as definitive diagnosis. The more specific myelodysplastic syndromes are: refractory anemia; refractory neutropenia; refractory thrombocytopenia; refractory anemia with ring sideroblasts; refractory cytopenia with multilineage dysplasia; refractory anemia with excess blasts; and refractory cytopenia of childhood. If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis. The peripheral blood and bone marrow are the principal sites of involvement.
The median age of patients with myelodysplastic syndrome is 70. Most patients present with symptoms related to cytopenia. Most patients are anemic and transfusion dependent. Occasionally there is neutropenia and/or thrombocytopenia.
About 10% of MDS patients have bone marrow that is hypocellular. These cases are referred to as hypoplastic MDS. When considering the diagnosis of hypoplastic MDS, it is important to exclude toxic myelopathy and auto-immune disorders.
This is a clinical diagnosis. When the testing has excluded other diseases, the physician uses the information from the equivocal test results plus the patient's clinical history to make a diagnosis of myelodysplastic syndrome.
The patient receives supportive care. Red blood cells are transfused for anemia.